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Reye’s Syndrome - Causes, Symptoms, Pathophysiology, Diagnosis, and Treatment

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Reye’s Syndrome - Causes, Symptoms, Pathophysiology, Diagnosis, and Treatment

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Reye's syndrome commonly affects children recovering from a viral infection. Read about its causes, symptoms, pathophysiology, diagnosis, and treatment.

Written by

Dr. Lochana .k

Medically reviewed by

Dr. Payas Joshi

Published At November 25, 2020
Reviewed AtAugust 11, 2023

Introduction:

Reye's syndrome is a severe condition that usually results in liver and brain swelling but can affect any organ in the body. It is a rare condition that commonly affects children recovering from a viral infection, such as the flu. The use of Aspirin is believed to be the cause, therefore, it is suggested to avoid giving Aspirin to children or teenagers for fever or headache. If the child develops confusion, loss of consciousness, or seizures, get them to the emergency room immediately. The child’s life can be saved by diagnosing and treating this condition early.

An Australian pathologist R. Douglas Reye was the first person to describe this syndrome in 1963, hence the name Reye’s syndrome. Although the use of Aspirin is approved for children over three years old, it should not be given to teenagers when they are recovering from a viral infection. In case the child has a fever or pain, Paracetamol (Acetaminophen) or Ibuprofen are safer options. Always consult the child’s pediatrician before giving him or her any medicine.

What Is Believed to Be the Cause of Reye’s Syndrome?

The cause of Reye's syndrome is still not clear. But researchers have identified some factors that are linked to this syndrome. It was observed that children who were treated with Aspirin for fever and pain associated with viral infections, especially chickenpox and flu, developed this syndrome.

It was also noticed that children and teenagers with fatty acid oxidation disorders, which is a group of hereditary metabolic disorders where the enzyme required to break down fatty acids is either missing or not functioning properly, were also affected more when given Aspirin.

Many infectious diseases have been linked to Reye’s syndrome. The viruses that increase the risk of Reye’s syndrome are:

  • Influenza A and B.

  • Varicella.

  • Parainfluenza.

  • Measles.

  • Adenoviruses.

  • HIV (human immunodeficiency virus).

  • Hepatitis A and B.

  • Rotavirus.

  • Coxsackie viruses.

  • Cytomegalovirus.

  • Epstein–Barr virus.

And infections caused by the following bacteria might also increase the risk:

  • Mycoplasma.

  • Shigella.

  • Salmonella.

  • Chlamydia.

This is an extremely rare condition, and the survival rate is around 80 percent.

What Is the Pathophysiology of Reye’s Syndrome?

Mitochondrial injury is thought to be the cause. When the mitochondria (the powerhouse of a cell) gets damaged, it inhibits oxidative phosphorylation and fatty-acid beta-oxidation. This might lead to the accumulation of ammonia, which results in the levels of ammonia and acidity in the blood to rise. The child’s blood sugar level might also drop. It also causes the brain and liver to swell up. The liver of deceased children appears fatty, and mitochondrial changes can be appreciated. The brain shows edema and other similar findings.

The salicylates present in Aspirin can damage the mitochondria, which gets intensified during a viral infection due to the release of endotoxins and cytokines, and leads to Reye’s syndrome. Some toxins and other medications are also said to increase the risk of this syndrome, which include:

  • Hypoglycin present in ackee fruit.

  • Expired Tetracycline.

  • Zidovudine.

  • Didanosine.

  • Aflatoxin.

  • Paint-thinner.

  • Valproic acid.

What Are the Signs and Symptoms of Reye’s Syndrome?

The signs and symptoms of this syndrome usually start around three to five days after the symptoms of a viral infection begin.

The early signs and symptoms are:

  • Diarrhea

  • Persistent vomiting

  • Rapid breathing

  • Lethargy or sleepiness.

The more serious signs and symptoms include:

  • Irritability.

  • Aggressive behavior.

  • Confusion.

  • Disorientation.

  • Weakness in hands and feet.

  • Loss of consciousness.

  • Hallucinations.

  • Seizures.

When to Consult a Doctor for Reye’s Syndrome?

Parents should identify the signs and symptoms of Reye’s syndrome promptly and seek help immediately. If the child is recovering from a viral infection, such as cold, flu, or chickenpox, and if he or she exhibits the following symptoms, consult a doctor immediately:

  • Seizures.

  • Lose consciousness.

  • Persistent vomiting.

How Does a Doctor Diagnose Reye’s Syndrome?

The doctor will conduct a complete physical examination and perform tests to rule out other conditions that can cause similar symptoms. The tests include:

  • Blood and urine tests - To detect the build-up of toxins or bacteria in the blood and to check how well the liver is functioning. They might also check for inherited metabolic disorders.

  • CT (Computed Tomography) of the Brain - To detect brain swelling.

  • Lumbar Puncture or Spinal Tap - Cerebrospinal fluid (CSF) is collected by inserting a needle with a syringe into the lower spine. This fluid is then tested for bacterial or viral infection.

  • Liver Biopsy - A sample of the liver is checked for any liver disease.

  • Skin Biopsy - A sample of the skin is taken to diagnose fatty acid oxidation disorders and other metabolic disorders.

What Is the Differential Diagnosis of Reye’s Syndrome?

Reye’s disease is extremely rare, and other conditions that can result in similar symptoms include:

  • Inherited metabolic disorders.

  • Meningitis - the inflammation of the membranes covering the brain and spinal cord.

  • Encephalitis - brain inflammation.

How Is Reye’s Syndrome Treated?

Reye’s syndrome is a medical emergency that needs prompt diagnosis and treatment. The child needs to be hospitalized. There is no specific treatment or cure for this condition, as the exact cause is not known. The child will be given supportive treatment that focuses on managing the symptoms and complications. The child might need:

  • Electrolytes and intravenous fluids to prevent dehydration and maintain electrolyte balance.

  • Insulin is administered to increase the metabolism of glucose.

  • Corticosteroids might be given to reduce brain swelling.

  • Diuretics to remove excess fluid.

  • Ammonia detoxicants to reduce the level of ammonia in the blood.

  • The child might be put on a ventilator if he or she has breathing problems.

  • Anticonvulsants are given to control seizures.

The doctor will closely monitor the liver and heart function. Late diagnosis of Reye’s syndrome can cause permanent brain damage and can also be fatal. Early diagnosis is crucial.

How Can Reye’s Syndrome Be Prevented?

Even though Aspirin is approved to be used in children above three years of age, avoid giving it to children and teenagers for fever and pain. Also, avoid medicines that contain Aspirin in combination with other drugs.

Ask the doctor to screen the newborn baby for fatty acid oxidation disorders. If the child tests positive for this disorder, then never give them Aspirin.

Make sure to check the medicine label before asking the child to take any tablet. The other names for Aspirin are:

  • Alka-Seltzer.

  • Acetylsalicylic acid.

  • Salicylic acid.

  • Salicylate.

  • Acetylsalicylate.

In addition to this, salicylate salts are found in some oral ulcer gels and dental gels. Children under the age of 16 should not be given these. Instead of Aspirin, one can use Paracetamol (Acetaminophen) or Ibuprofen for fever and pain.

Note - Children with Kawasaki disease or other chronic diseases might need prolonged treatment with medicines that contain Aspirin. In case the child needs to take Aspirin, make sure his or her chickenpox and flu vaccine is up-to-date.

What Is the Prognosis of Reye’s Syndrome?

The survival rate is around 80 percent. The prognosis is bad if the child is in a coma. In some cases, this syndrome can result in complications like:

  • Bad memory.

  • Poor attention span.

  • Partial loss of vision.

  • Some degree of hearing loss.

  • Speech difficulties.

  • Movement problems.

  • Swallowing difficulties.

Conclusion:

To conclude, Reye's syndrome is a very uncommon condition that can seriously harm the brain and kidneys in children. If it is not treated right away, it could cause lasting brain damage or even death. As the use of Aspirin is linked to this syndrome, one should never self-medicate their child without a pediatrician’s supervision.

Frequently Asked Questions

1.

What Happens in Reye's Syndrome?

Reye's syndrome is a serious condition but a rare disorder that occurs in children and adolescents. It usually occurs in children recovering from a viral infection, resulting in swelling in the liver (hepatitis) and brain (encephalopathy).

2.

Which Organs Are Affected by Reye's Syndrome?

Reye's syndrome affects many organs, but the most commonly affected organs are the liver and brain.

3.

Can Reye's Syndrome Go Away on Its Own?

Reye's syndrome cannot go away on its own because there is no cure for it, but the symptoms can be managed. Steroids can be used to reduce swelling in the brain. Experts are not sure what causes this syndrome, but the more clear evidence is that it occurs in children taking Aspirin who are recovering from a viral infection.

4.

Can Reye's Syndrome Be Fatal?

Even though most of the children and teenagers with Reye's syndrome can survive, it can also cause permanent brain damage. Without proper diagnosis and treatment, Reye's can be fatal within a few days.

5.

How Severe Is Reye's Syndrome?

Reye's syndrome is a rare but severe condition that can cause liver and brain damage. Without proper treatment, it can be fatal within a few days.

6.

Can Reye's Syndrome Spread From Person to Person?

Reye's syndrome is not a contagious disease, but it is often noticed in children recovering from viral infections like flu or chickenpox.

7.

What Is the Best Method to Prevent Reye's Syndrome?

Reye's syndrome has become less common because most doctors and parents do not give Aspirin to children who are recovering from a viral infection. It is better to give Acetaminophen (Paracetamol) or Ibuprofen if there is any pain instead of Aspirin.

8.

Can Reye's Syndrome Cause Fatty Changes in the Liver?

Reye's syndrome's most common liver-related complications are abnormal liver function, fatty deposits in the liver, and poor bleeding and clotting caused by liver failure.

9.

When Was Reye's Syndrome Discovered?

Reye's syndrome was discovered by an Australian pathologist R. Douglas Reye in 1963. Therefore it is named Reye's syndrome.

10.

How to Test for Reye's Syndrome?

The test used to diagnose Reye's syndrome are:
- Spinal tap (Lumbar puncture).
- Liver biopsy.
- Skin biopsy.
- CT (computed tomography) scan of the head.
- MRI (magnetic resonance imaging) scan of the head.

11.

What Can Be the Standard Treatment for Reye's Syndrome?

The standard treatment for Reye's syndrome usually involves hospitalization, but in severe cases, the doctor may send the children to the intensive care unit (ICU). The other treatment includes:
- Diuretics to treat increased fluid loss due to urination and intracranial pressure.
- Giving intravenous fluids like glucose and electrolytes.
- Doctors can provide medications like Vitamin K, plasma, and platelets to treat bleeding due to liver abnormalities.
- Cooling blankets can be used in patients to maintain internal body temperature.

12.

Can Adults Be Affected by Reye's Syndrome?

Reye's syndrome can occur in people of any age group, but it is most commonly seen in children and teenagers who take Aspirin while recovering from a viral infection.
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Dr. Payas Joshi
Dr. Payas Joshi

Pediatrics

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